Giant cell tumor of bone is a neoplasm that is rarely seen in children. Goltz syndrome is a disorder that affects multiple ectodermal and mesodermal tissues and has occasionally been associated with giant cell tumors of bone. Our case of giant cell tumor in a 5-year-old girl with Goltz syndrome suggests that this syndrome provides a unique situation wherein the practitioner should consider giant cell tumor of bone, even in a pediatric setting.
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